Paediatric ophthalmology: Case ten

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This 2 year-old presented to the ophthalmologist with the above facial appearance. There was no history of trauma and the blood test including clotting screen was normal.

a. What is the differential diagnosis?

The picture shows bilateral orbital ecchymosis, the possible diagnoses include:

• non-accidental injury
• clotting abnormality such as idiopathic thrombocytopaenia purpura
• metastatic neuroblastoma in the orbit

b. Further examination revealed bilateral proptosis. What is the most likely diagnosis?

The combination of ecchymosis and proptosis strongly suggest the possibility of metastatic neuroblastoma to the orbits.

Neuroblastoma is a common childhood malignancy. It originates from the embryonic neuroblastic tissues within the sympathetic nervous system within the adrenal gland, thorax or chest. Orbital metastasis occurs in about 20% of cases causing proptosis and ecchymosis.

c. What other ocular signs may occur with the condition mentioned in b.?

Other ocular signs include:

• Horner's syndrome occurs with neuroblastoma of the cervical chain
• opsoclonus in which the eyes show multidirectional saccadic eye movement. It occurs with localized neuroblastoma as a paraneoplastic sign.

d. What is the prognosis for this patient?

Presence of orbital neuroblastoma is associated with poor prognosis. The survival rate is about 15% even with intensive treatment such as radiation and chemotherapy.

The prognosis of neuroblastoma is affected by:

• age of the patient (better in those less than one year of age)
• site of the lesion (better with lesion arising from chest than abdomen)
• presence of metastasis (better with localized lesion)